Brittle cornea syndrome is a form of Ehlers-Danlos syndrome characterized by a severe ocular manifestations due to extreme corneal thinning and fragility with rupture in the absence of significant trauma, and progression to blindness. Extraocular manifestations comprise deafness, developmental hip dysplasia, and joint hypermobility. The disease is Monarch Disease Ontology entry MONDO_0009242 (brittle cornea syndrome). Also known as: brittle cornea syndrome type 1, kyphoscoliosis type, type VIB Ehlers-Danlos syndrome.